Abstract: TH-PO699
A Unique Case of IgA Nephropathy Associated with Both Immune Thrombocytopenia and Erythrocytosis
Session Information
- Glomerular Diseases: Case Reports - 1
October 24, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Lehmann, Parker, Idaho College of Osteopathic Medicine, Meridian, Idaho, United States
- Bhatt, Udayan Y., The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
- Thakkar, Asish, VA Health Care System, Columbus, Ohio, United States
Introduction
Erythrocytosis is characterized by an elevation in hemoglobin and may be primary (typically associated with a mutation in the JAK2 gene) or it may be secondary. Many renal diseases have been associated with erythrocytosis. One association that has become increasingly reported is the association between IgA nephropathy and erythrocytosis. In addition, there have been a few case reports of IgA nephropathy associated with immune thrombocytopenia (ITP). We describe a novel presentation of both erythrocytosis and immune thrombocytopenia associated with IgA nephropathy.
Case Description
A 26yo African American male presented to the Hematology clinic for mild thrombocytopenia. The patient had a positive platelet associated antibody. This resulted in the diagnosis of ITP. The patient was coincidentally noted to have chronic kidney disease with eGFR of 65cc/min at that time. He subsequently had a climbing hemoglobin and began requiring therapeutic phlebotomy. His erythrocytosis showed no evidence of a JAK2 mutation and a bone marrow biopsy showing a normocellular marrow with an erythroid predominant hematopoiesis but normal megakaryocytes. The diagnosis was idiopathic erythrocytosis. At 36yo, repeat chemistries showed an eGFR of 33cc/min. Because of that, he was referred to nephrology and underwent a renal biopsy that showed IgA nephropathy with moderate interstitial fibrosis and tubular atrophy but without active injury or thrombotic microangiopathy. The patient continued to receive phlebotomy. He was noted to have a normal to occasionally elevated erythropoietin level. Imaging did not show evidence of intrabdominal or renal neoplasms. The patient has not manifested any hemorrhagic complications and his platelet count has continued to be mildly suppressed.
Discussion
IgA nephropathy is the most common primary glomerular disease worldwide. It is diagnosed via biopsy and there are no exact immune markers that definitively suggest IgA nephropathy. However, the presence of hematologic disorders such as ITP and erythrocytosis may also indicate the presence of underlying IgA nephropathy. Our unique case describes IgA nephropathy associated with both ITP and erythrocytosis. Further work is warranted in exploring the pathogenesis of these connected disorders.